natriuretic-peptide--brain and Sarcoidosis--Pulmonary

Pulmonary hypertension (PH) is a well-recognized complication of sarcoidosis. Patients with sarcoidosis-associated PH (SAPH) have poorer functional status and greater supplemental oxygen requirements than sarcoidosis patients without PH, and are more likely to be listed for lung transplantation. PH is an independent risk factor for mortality in sarcoidosis patients awaiting lung transplantation. The pathophysiology of SAPH is complex, with multiple mechanisms contributing to pathogenesis, including the fibrous destruction of the pulmonary vascular bed, extrinsic compression of the central pulmonary vessels and an intrinsic vasculopathy. Recognition of SAPH may be delayed as it can be masked by the clinical picture of underlying pulmonary sarcoidosis, and right heart catheter remains the gold-standard for diagnosis. Management of SAPH is based on reversal of resting hypoxaemia, treatment of comorbidities and treatment of the underlying sarcoidosis. The use of corticosteroids in SAPH is controversial. Specific PH therapy is not routinely recommended in SAPH as there are no successful placebo-controlled trials, although there is limited data to suggest that endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be useful.

Topics: Cardiomyopathies; Endothelin-1; Humans; Hypertension, Pulmonary; Lung; Natriuretic Peptide, Brain; Sarcoidosis, Pulmonary; Ultrasonography; Vasoconstriction

Pulmonary hypertension (PH) is a feared complication in patients with sarcoidosis.It is an important negative prognostic factor which is reflected as a priority given to these subjects for orthotopic lung transplantation. We evaluated the relationship between BNP, 6MWD test, DLCO% values and the severity of sarcoidosis-associated pulmonary hypertension.. A retrospective chart review was conducted between January 2010 and January 2015 on consecutive confirmed adult subjects diagnosed with sarcoidosis. Demographics, medical comorbidities, clinical features, pulmonary function tests (PFTs), 6MWD test, transthoracic echocardiography, laboratory investigations, radiographic findings, treatment and outcome data were collected from medical records. Subjects with suspicion for PH (i.e. those with PASP ≥ 25 mmHg) were included in the study. PH severity was classified using PASP into mild (PASP 25-49 mmHg), moderate (50-69 mmHg) and severe (≥70 mmHg). We evaluated the strength of correlation between BNP, 6MWD test, DLCO% and the severity of PH. The association between variables was performed using Pearson correlation coefficient and results were considered statistically significant if P value was <0.05.. Among the 108 cases diagnosed with sarcoidosis, we identified a total of 27 patients (25%) who had an elevated PASP suggestive of PH and met the study inclusion criteria. There was a significant correlation between BNP level (r=0.804, P=0.003), 6MWD test (r=-0.865, P=0.000), DLCO% (r=-0.513, P=0.015) and the PASP in sarcoidosis patients.. Although these simple tests should not be used as screening tools for suspecting sarcoidosis-associated pulmonary hypertension, they may be of value in following its progression in subjects already diagnosed with pulmonary hypertension.

Topics: Aged; Biomarkers; Disease Progression; Exercise Test; Exercise Tolerance; Female; Humans; Hypertension, Pulmonary; Male; Middle Aged; Natriuretic Peptide, Brain; Predictive Value of Tests; Prognosis; Pulmonary Diffusing Capacity; Retrospective Studies; Sarcoidosis, Pulmonary; Severity of Illness Index; Time Factors; Walking

Pulmonary hypertension (PH), an increasingly recognised complication of pulmonary sarcoidosis, is associated with increased morbidity and mortality Evidence of benefit with targeted therapies in sarcoidosis associated pulmonary hypertension (SAPH) is limited.. We conducted a retrospective review of patients with sarcoidosis and right heart catheter proven PH who received treatment with targeted therapies (phosphodiesterase-5 inhibitors, endothelin receptor antagonists, or combination) at our hospital. Six minute walk test (6MWT), World Health Organisation (WHO) functional class, echocardiography, pulmonary function test (PFT) and serum brain natriuretic peptide (BNP) data were collected at baseline and during follow-up.. Thirty-three patients (16 men) with a mean age of 55.5 ± 10.7 years and mean pulmonary artery pressure of 44.0 ± 8.6 mm Hg received treatment with targeted PH therapies (sildenafil=29, bosentan=4). At six months, median six minute walk distance improved from 227 (88-526) meters to 240 (140-380) metres (p=0.04), median serum BNP levels improved from 35 (2-424) pmol/L to 26 (4-255) pmol/L (p<0.01), and at echocardiography, median tricuspid annular plane systolic excursion (TAPSE) improved from 17.5 (8.0-27.0) mm to 20.0 (15.0-27.0) mm (p=0.03). WHO functional class improved in 14 patients. Two patients developed side-effects attributed to sildenafil (n=1) or bosentan (n=1), requiring conversion to alternative PH therapies. Ten patients died, and one patient underwent lung transplantation, a median of 13.5 (3-37) months after commencing targeted therapies.. Our results suggest that targeted therapies are safe in patients with SAPH. Controlled trials are warranted before therapeutic recommendations can be made.

Topics: Aged; Antihypertensive Agents; Arterial Pressure; Biomarkers; Endothelin Receptor Antagonists; Exercise Test; Exercise Tolerance; Female; Humans; Hypertension, Pulmonary; London; Lung Transplantation; Male; Middle Aged; Molecular Targeted Therapy; Natriuretic Peptide, Brain; Phosphodiesterase 5 Inhibitors; Recovery of Function; Retrospective Studies; Sarcoidosis, Pulmonary; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Vasodilator Agents

We sought to examine whether the plasma brain natriuretic peptide (BNP) levels are elevated in the cardiac sarcoidosis patients even with a preserved ejection fraction. The data from the patients with either pulmonary sarcoidosis without any evidence of cardiac involvement (n = 13) or cardiac sarcoidosis (n = 8) with a preserved ejection fraction (>55%) on echocardiography were analyzed. The median plasma BNP levels were significantly higher in the patients with cardiac sarcoidosis than in those with pulmonary sarcoidosis (101.5 +/- 65.1 vs. 15.6 +/- 10.5 pg/ml, p < 0.001), although there was no significant difference in left ventricular ejection fraction between the two populations. The plasma BNP level is thus considered to be a useful non-invasive biomarker for identifying a possible cardiac involvement in the sarcoidosis patients with a preserved ejection fraction.

Topics: Adult; Aged; Biomarkers; Cardiomyopathies; Female; Humans; Male; Middle Aged; Natriuretic Peptide, Brain; Sarcoidosis; Sarcoidosis, Pulmonary; Stroke Volume

Electrocardiographic conduction abnormalities including development of atrioventricular block, bundle branch block or ventricular arrhythmias are characteristic manifestations of cardiac sarcoidosis (CS). The present study seeks to show the minute conduction abnormality by detection of late potentials (LP) on signal averaged electrocardiogram (SAECG).. Ten patients with CS, 52 patients with pulmonary sarcoidosis (PS) but no obvious cardiac manifestations and 52 normal controls were studied. All participants underwent SAECG to detect LP. In patients with CS (the CS group), LP were detected in 8 patients (80%). In 52 patients with PS, LP were detected in 25 patients (46.2%, PS-LP(+) group), comparing only 3 (5.8%) of normal controls (p<0.0001). The remaining 27 patients with PS with negative LP were classified in the PS-LP(-) group. In the CS group, premature ventricular contraction frequency on Holter's monitoring and plasma B-type natriuretic peptide concentrations were significantly higher than those in the PS group. However, no significant difference in these parameters between PS-LP(+) and PS-LP(-) groups were found.. In the PS patients without obvious cardiac manifestations, LP were detected as high as 46.2%, suggesting latent minute conduction abnormality. The higher incidence of LP in PS might be considered as an expression of latent myocardial fibrosis. Close follow-up is needed in these patients.

Topics: Action Potentials; Adult; Aged; Arrhythmias, Cardiac; Electrocardiography; Female; Fibrosis; Heart; Heart Conduction System; Humans; Male; Middle Aged; Myocardial Contraction; Natriuretic Peptide, Brain; Peptidyl-Dipeptidase A; Sarcoidosis, Pulmonary